Data in Brief 4 (2015) 614–621

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Data Article

Micronutrient in hyperphenylalaninemia Vanesa Crujeiras a, Luís Aldámiz-Echevarría b, Jaume Dalmau c, Isidro Vitoria c, Fernando Andrade b, Iria Roca d, Rosaura Leis a, Ana Fermandez-Marmiesse e, María L. Couce e,n a Unit of Gastroenterology and Nutrition, Department of Pediatrics, Hospital Clinico Universitario de Santiago, Travesía da Choupana s/n, 15706 Santiago de Compostela, A Coruña, Spain b Unit of Metabolism, Department of Pediatrics, Hospital de Cruces, Group of Metabolism, Biocruces Health Research Institute, CIBERER, Plaza de Cruces s/n, 48903 Barakaldo, Vizcaya, Spain c Unit of Metabolopathies, Hospital Universitario la Fe, Bulevarsur s/n, 46021 Valencia, Spain d Unit of Diagnosis and Treatment of Congenital Metabolic Diseases, S. Neonatology, Department of Pediatrics, Hospital Clinico Universitario de Santiago, Travesía da Choupana s/n, 15706 Santiago de Compostela, A Coruña, Spain e Unit of Diagnosis and Treatment of Congenital Metabolic Diseases, S. Neonatology, Department of Pediatrics, Hospital Clinico Universitario de Santiago, CIBERER, Health Research Institute of Santiago de Compostela (IDIS), Travesía da Choupana s/n, 15706 Santiago de Compostela, A Coruña, Spain

a r t i c l e in f o

a b s t r a c t

Article history: Received 8 July 2015 Received in revised form 17 July 2015 Accepted 20 July 2015 Available online 1 August 2015

The data presented here are the biochemical parameters of 156 patients with hyperphenylalaninemia. PKU patients, who, in order to maintain optimal serum Phe concentrations, receive dietary treatment consisting of a diet low in natural protein supplemented with special low protein foods and a Phe-free amino acid mixture, vitamins and minerals. The obtained data reflects a high percentage of patients with prealbumin and selenium deficiencies, as well as an increased level of folic acid. This data article is related to the research article entitled, “Vitamin and mineral status in patients with hyperphenylalaninemia”, by Crujeiras et al. [1]. & 2015 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).

DOI of original article: http://dx.doi.org/10.1016/j.ymgme.2015.06.010 Corresponding author. E-mail addresses: [email protected] (L. Aldámiz-Echevarría), [email protected] (J. Dalmau), [email protected] (I. Vitoria), [email protected] (F. Andrade), [email protected] (I. Roca), [email protected] (R. Leis), [email protected] (A. Fermandez-Marmiesse), [email protected] (M.L. Couce). n

http://dx.doi.org/10.1016/j.dib.2015.07.026 2352-3409/& 2015 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).

V. Crujeiras et al. / Data in Brief 4 (2015) 614–621

615

Specifications Table Subject area More specific subject area Type of data How data was acquired Data format Experimental factors Experimental features Data source location Data accessibility

Metabolic diseases Vitamins and minerals in phenylketonuria Table By clinical history Analyzed data None applied -Tandem mass-spectrometry and standard procedures of laboratory PKU patients from three Spanish Centers: Hospital Universitario La Fe-Valencia, Hospital Universitario de Cruces-Basque Country and Hospital Clínico Universitario de Santiago-Galicia. Data is supplied in this article

Value of the data 

A high percentage of selenium deficits as well as a high percentage of folic acid overloads are detected in PKU patients treated, which should lead us to reflect on whether to adjust international standards supplements formulated milks.

1. Data, experimental design, materials and methods The population comprised PKU patients from three Spanish Centers: Hospital Universitario La FeValencia, Hospital Universitario de Cruces-Basque Country and Hospital Clínico Universitario de Santiago-Galicia. Patients were followed in their center from diagnosis to date. We included both patients diagnosed by newborn screening as well as those diagnosed later. Those who underwent sporadic monitoring were excluded, as well as those cases in which informed consent was not signed by parents and/or patients. The variables collected for each patient were: age, sex, phenotype (mild hyper-Phe (MHPA) 120– 360 μmol/L; mild-moderate PKU 360–1200 μmol/L (MPKU); classic PKU41200 μmol/L (CPKU) (according to US Guidelines [2], disease detection (early and late diagnosis), annual median blood Phe levels (target blood Phe levels considered adequate for children under 6 years of age were o360 mmol/L; o480 mmol/L between 6 and 10 years and r600 mmol/L in older patients), anthropometric measurements (weight, height and BMI), phenylalanine tolerance and BH4 treatment in the BH4 responsive patients; blood chemistry (total protein, prealbumin, electrolytes, selenium (Se), zinc (Zn), B12, folic acid, ferritine, 25hydroxy vitamin D (25-OHD). Micronutrients levels evaluated present the following reference intervals (valid for any patient age): Total protein: 6.3–8.5 g/dL; calcium (Ca): 8.7–10.8 mg/dL; phosphorus (P): 2,6-5.9 mg/dL, prealbumin: 21–41 mg/dL; vitamin B12: 180–1900 pg/mL; folic acid: 3–17.5 ng/mL; 25-OHD: 20–42 ng/mL; ferritin: 14–325 ng/mL; Zn: 65–140 mg/dL; Se: 60–125 mg/L

2. Data In PKU patients, micronutrient deficiencies but no clinical symptoms have been reported, mainly for Zn, Se, Fe, folate and B12 [3–5]. In this study 127 patients had altered values in one or several markers evaluated (Table 1), 58.33% were from CPKU, 21.67% from MPKU and 20% from MHPA patients. Interestingly, from these patients with altered values, the 84.16% had adequate adherence to diet.

616

Table 1 Characteristics of Hyperphenylalaninaemia patients with values of the biochemical parameters studied. Prot g/ dL

Prealb mg/ dL

Ca mg/ dL

P mg/ dL

Fer ng/ mL

B12 pg/ mL

Folic ng/ mL

25-OHD ng/ mL

Zn mg/ dL

Se mg/ dL

345 217 223 218 469 367 118 454 243 390 289 379 440 153 1162 473 447 205 242 194 432 448 503

138 380 258 123 408 1032 162 480 90 474 225 207 522 300 336 348 450 777 330 204 366 243 252

6.6 7.9 7 6.4 7.5 7.5 6.5 7.7 6.7 6.8 7 6.8 6.6 7.5 6.7 6.4 7.7 6.5 6.9 6.8 6.9 6.6 7.6

18↓ 25 17↓ 14↓ 30 36 14↓ 28 16↓ 22 14↓ 17↓ 34 19↓ 21 22 19↓ 31 30 21 18↓ 20↓ 26

9.9 9.8 10 10.2 10.1 10.2 9.2 10.4 10.1 9.6 10.3 10 9.3 10.1 10.4 9.8 10 9.4 9.3 10.1 10.1 10.1 9.9

5.6 3.3 5.6 5.6 3.9 4 4 4 5.2 5.2 4.7 5.1 3.9 4.9 4.5 5 4.6 2.3↓ 4.5 9.9↑ 3.8 4.8 4.8

135 36 29 30 37 32 84 126 28 5↓ 40 28 40 52 34 21 32 89 40 24 48 32 9.9↓

853 823 789 444 723 272 696 405 934 347 835 462 980 617 779 468 388 354 761 560 421 656 824

24↑ 24↑ 15.1 13 24↑ 8.6 18.7↑ 17.3 18.5↑ 24↑ 13.2 17.2 20↑ 16.9 18.6↑ 24↑ 5.68 16.5 12 – 16.7 20.2↑ 16.9

40 38.7 40.2 26 37.4 25.5 36.2 19.1↓ 40 34.4 29.3 27.8 41.3 26.4 48↑ 40.6 27.2 27.6 24 34.2 28.7 31.9 36.9

99 117 76.5 56.6↓ 78.5 119 81.5 70 78.5 82.5 59.7↓ 106 145↑ 71.5 76.5 76.5 101 113 80 78.5 65 78.5 103

47↓ 74 106 38.6↓ 32.3↓ 52.7↓ 68.7 70 62.5 69.5 19.6↓ 62 69.5 57↓ 68.7 58.7↓ 71 51.7↓ 36.1↓ – 57↓ 34↓ 68.7

E

320

381

7.4

28

9.3

3.5

61

628

16.3

41.3

144↑

68.7

E E E E E E E L E E E E

381 409 425 771 226 256 250 169 55 537 403 489

444 1158 258 972 192 846 90 576 156 156 138 384

6.1↓ 7.7 7.2 6.4 6.3 7.2 6.4 6.8 6.4 7.3 6.5 6.7

19↓ 21 16↓ 21 16↓ 37 20↓ 22 17↓ 20↓ 18↓ 19↓

9.8 9.6 9.6 8.5↓ 10.7 10.1 10.3 9.8 9.8 10.4 9.9 10

5.9 3 5 2.7 5.9 2.3↓ 6.4↑ 3.3 3.3 5.5 5.1 4.5

97 39 13↓ 22 8↓ 91 267 23 127 42 24 27

838 524 675 301 679 471 1201 937 423 600 372 1875

24↑ 6.4 15.1 11.5 16.3 14.4 17.2 24↑ 16 14.3 16.9 18.5↑

32.2 30.4 19↓ 20.2 36.5 41.9 37.5 20 31.8 42 52↑ 43.2↑

125 120 73.5 75.5 80.5 145↑ 170↑ 75.5 93 71.5 74.5 77

68.7 40.18↓ 74.9 – 68.7 77.2 77.2 49.4↓ 71 135↑ 124 54.8↓

Sex

Age

BMI

Diag

Tre. D/ TD Phe tol mg/ T d

1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23

F M M F M M F F F F F M F F M M F M M M F M M

N ↑ N ↑↑ N ↑ ↑ N ↓ ↑↑ N N ↑ N N ↓ N ↑ ↑↑ ↑↑ ↑ N N

CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU

D D D D D D D D D D D D D D D/T D D D D D D D D

E L E E E E E E E E E E E E E E E E E E E E L

24

F



CPKU

D

25 26 27 28 29 30 31 32 33 34 35 36

M F M F M M M F M F M M

2y 11m 19y 3m 2y 7m 2y 7m 18y 2m 28y 9m 1y 2m 15y 2m 2y 4m 13y 1m 3y 3m 4y 10m 92y 7m 9y 6m 7y 2y 6m 15y 6m 39y 5y 8m 3y 2m 14y 9m 2y 9m 18y 10m 23y 11m 3y 9m 34y 9m 5y 10m 30y 4m 3y 7m 23y 9m 17y 4m 10y 6m 6y 4m 5y 11m 5y 1m

↑ ↑↑ ↑ N N ↑ N ↑↑ N N ↑ N

CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU CPKU

D D D D D D D D D D D D

V. Crujeiras et al. / Data in Brief 4 (2015) 614–621

Phe median mM

P

M F F M M F M M M F F F M M

51 52

F M

53 54 55

F F F

56 57 58

M M F

59 60 61

M M F

62 63

F F

64 65 66 67 68 69 70 71 72 73

M M F F F F M M F F

↑ ↑↑ N ↑ N N ↑↑ N N ↑ N N N N

CPKU MPKU MPKU MPKU MPKU MPKU MPKU CPKU CPKU CPKU MPKU MPKU CPKU CPKU

D D/T D D D D D D D D D D D D

L E E E E E E L E L E E E L

121 1069 438 430 312 1521 435 401 360 438 587 245 552 299

888 198 117 300 150 882 174 663 294 516 132 192 264 897

7.6 6.7 7 7 7.2 7.5 7 7.8 6.7 6.9 6.9 7.1 7.2 7.2

24 18.4↓ 22.8 19.1↓ 22.6 42↑ 26.3 25.7 14.5↓ 26.6 20.2↓ 17.2↓ 29.1 34

9.2 10 10.2 9 9.9 10.2 10.2 9.4 9.7 9.4 9.6 9.8 9.9 9.8

– – – – – – – – – – – – – 3.5

73 35 34 108 30 13 – 11↓ 15 – – – 23 52

660 501 766 581 669 387 967 2000↑ 737 368 290 582 616 302

30↑ 30↑ 28↑ 9.44 21.1↑ 21.6↑ 17.6↑ 28↑ 28↑ 18.45↑ 13.72 24↑ 24↑ 13.4

26 33 36 17↓ 38 33 27 29 39 30 28 21 25 16.1↓

69.2 87.3 67.2 76.8 108 95 98 71 73 75 68 65 85 59↓

60 42↓ 48↓ 70 44↓ 73 79 84 66 56↓ 73 58↓ 64 50↓

N ↑

CPKU CPKU

D D

L L

765 600

751 816

7.5 7.6

29 26

9.6 10

2.6 3.1

15 40

482 698

43.9↑ 28.9↑

20 27

73 77

70 58↓

N ↑↑ N

CPKU MPKU CPKU

D D D

L L E

692 478 450

300 766 490

7.3 7.1 6.7

25 24 20↓

9.3 10.1 9.8

2.9 3.2 3.3

22 42 25

1767 401 258

32.5↑ 24↑ 17.9↑

27 22 11↓

74 84 70

54↓ 67 52↓

↑ ↑ N

CPKU CPKU CPKU

D D D

L L L

505 962 481

696 422 679

7.9 7.6 7.8

23 32 26

9.8 10.4 9.9

2.7 2.6 2.8

21 82 23

730 1031 832

22.01↑ 49.6↑ 32.6↑

17↓ 28 24

71 98 71

53↓ 122 83

↑↑ ↑ ↑

CPKU MPKU CPKU

D D/T D

E E E

800 229 730

695 452 388

7.6 7.7 7.2

36 34 28

10.2 9.9 10

2.7 3.6 2.3↓

114 203 22

495 571 391

38.8↑ 30.6↑ 19.7↑

18↓ 21.5 44↑

85 84 63↓

83 86 58↓

N ↑↑

CPKU CPKU

D D

E E

237 511

431 370

7.5 7.3

28 29

9.8 10.2

2.6 4.6

18 90

1266 435

22.2↑ 31.1↑

17↓ 30

79 66

82 77

↑ N N ↑↑ ↑ ↑ N ↓ N ↑↑

CPKU CPKU MPKU MPKU MPKU MPKU MPKU MPKU MPKU CPKU

D D D/T D/T D D/T D D/T D/T D

E E E E E E E E E E

312 700 3051 461 2212 802 1500 2585 240 314

613 567 262 197 437 261 680 231 277 427

7.7 6.4 7.2 7.7 8 7.3 7.6 7.3 7.1 7.8

29 20↓ 37 28 33 44↑ 31 20↓ 29 29

10.2 10.4 9.9 10.3 10.1 10.4 10.5 10.2 9.9 10.1

7.7↑ 3.2 4 3.7 3.4 3.3 3 4.8 3.5 4

246 – 32 14 37 21 46 40 10↓ 45

600 337 584 419 605 407 521 589 476 725

16.5 7.9 20.6↑ 10.4 29.1↑ 5.9 8.5 16.7 21.1↑ 9.7

27 33 31 17↓ 19.2↓ 31 25 21 24 23

101 104 67 75 74 96 76 74 100 92

45↓ – 76 87 71 70 51↓ 69 86 45↓ 617

42y 6m 5y 5m 4y 1m 26y 4m 3y 19y 9m 3y 36y 4y 6m 43y 1m 12y 1m 8y 5m 9y 4m 44y 11m 41y 8m 39y 11m 39y 6m 38y 2m 34y 11m 32y 2m 32y 1m 31y 11m 30y 7m 28y 6m 24y 11m 23y 8m 20y 11m 20y 9m 19y 4m 19y 7m 19y 3m 18y 8m 17y 4m 16y 8m 15y 4m 15y 9m 14y 10m

V. Crujeiras et al. / Data in Brief 4 (2015) 614–621

37 38 39 40 41 42 43 44 45 46 47 48 49 50

618

Table 1 (continued ) Sex

Age

BMI

Diag

74 75 76 77 78 79 80 81 82 83 84 85 86 87 88 89 90 91 92 93 94 95 96 97 98 99 100 101 102 103 104 105 106 107 108 109 110 111 112

F F M F M M M M F M M F F M F F F M F M F M M M F F F F F F M M F M F M F F F

14y 8m 14y 5m 13y 5m 12y 8m 10y 6m 11y 5m 11y 4m 11y 9y 9m 8y 7y 10m 7y 8m 7y 8m 7y 2m 5y 7m 5y 6m 4y 3y 8m 1y 10m 1y 4m 1y 4m 9m 7m 1y 11m 5y 8m 15y 2m 6y 7m 9y 8y 4y 8m 4y 1y 1m 9y 4y 3y 11m 1y 1m 7y 10m 6y 3m 4y 1m

N N N ↓ ↑ ↑↑ ↑↑ N ↑↑ N ↑↑ ↑↑ ↑↑ N ↑ N N N N N ↑↑ ↑ N N N N ↑ N ↑ N ↑ N N ↑↑ ↓ N N N N

CPKU MPKU CPKU CPKU CPKU CPKU MPKU CPKU MPKU MPKU CPKU MPKU MPKU CPKU MPKU CPKU MPKU MPKU CPKU CPKU CPKU CPKU CPKU HPA HPA HPA HPA HPA HPA HPA HPA HPA HPA HPA HPA HPA HPA HPA HPA

Tre. D/ TD Phe tol mg/ T d D D/T D D D D D D D D/T D D/T D/T D D/T D D/T D D D D D D – – – – – – – – – – – – – – – –

E E E E E E E E E E E E E E E E E E E E E E E E E E E E E E E E E E E E E E E

2000 513 260 399 261 521 524 148 1250 352 872 900 457 815 380 1050 264 165 174 319 259 222 750 2601 3000 2700 2600 2400 1900 2800 600 2000 1450 1350 857 1500 722 750 2000

Phe median mM

Prot g/ dL

Prealb mg/ dL

Ca mg/ dL

P mg/ dL

Fer ng/ mL

B12 pg/ mL

Folic ng/ mL

25-OHD ng/ mL

Zn mg/ dL

Se mg/ dL

388 199 227 363 430 310 274 191 196 202 171 161 155 160 266 162 195 179 93 78 98 222 204 250 258 280 240 270 230 225 180 127 210 300 305 320 230 173 175

8.3 8 7.8 7.7 7.1 7.6 7.7 7 7.3 6.8 6.4 7.3 7.1 7 6.6 7.7 6.9 7 7.1 7 6.3 6.8 6.8 6.9 7.5 8 7.1 7.4 7.5 6.8 6.9 6.5 6.9 7.3 7.3 6.6 7.2 7.1 6.5

34 27 18↓ 25 23 23 34 19↓ 23 20↓ 15↓ 19↓ 18↓ 23 20↓ 14↓ 20↓ 15↓ 13↓ 25 16↓ 14↓ 21 18↓ 21 23 27 20↓ 24 20↓ 22 23 22 16↓ 16↓ 20↓ 15↓ 16↓ 16↓

9.8 9.8 10.1 10.1 10.3 10.8 11↑ 10.2 10.4 10.1 9.2 9.8 9.8 9.5 9.9 10 10.4 9.7 10.1 10.2 10.1 10.7 10.6 10.2 9.9 9.8 9.6 10.1 10.2 9.6 9.7 11.3↑ 9.7 9.9 9.7 9.5 10 9.9 9.5

4.1 3.3 4 4.5 3.8 5 3.2 4.3 4.2 5.1 4.3 4.3 4.5 4 5.3 4.1 5.7 4.2 5.3 5.5 5.5 5.6 5.7 4.9 4.5 3.6 5.1 4.8 4.1 4.7 5 5.7 4.8 5.1 4.7 5.1 4.3 5.2 4.6

21 17 43 39 29 17 23 32 75 29 39 35 46 28 37 83 6↓ 42 27 29 18 25 22 23 18 6↓ 22 57 21 24 12↓ 23 21 9↓ 14 41 43 26 42

959 583 1189 911 1060 920 598 1192 541 788 687 833 865 1134 253 1293 748 868 947 774 548 1882 623 290 545 730 542 474 638 738 547 627 720 488 796 727 1108 686 905

20.5↑ 12.9 11.1 31↑ 32.4↑ 22.9↑ 19.8↑ 16.2 30.4↑ 17.5 24↑ 34.1↑ 30.8↑ 38.4↑ 8.7 32.5↑ 11.5 36.8↑ 26.9↑ 30.7↑ 23.4↑ 17.5 21↑ 10.1 13.4 – 10.8 18.8↑ 17.5 13.6 18.6↑ 6.4 19↑ 4.1 17.5 23.8↑ 9 11.9 19.1↑

23 18↓ 33 28 31 33 42 27 38 36 29 26 46↑ 14↓ – 25 26 24 22 19↓ 27 32 25 18.7↓ 17↓ 29 17.3↓ 30 18↓ 31 21 34 18↓ 27 33.9 17.6↓ 28 16.6↓ 33

105 75 83 86 80 74 94 94 83 73 92 76 74 91 95 91 66 67 98 110 113 131 91 73 82 74 68 67 101 88 110 88 75 73 57↓ 101 60↓ 87 72

74 71 74 71 54↓ 49↓ 51↓ 84 75 63 56↓ 69 72 63 68 86 75 49↓ 59↓ 52↓ 40↓ – 50↓ 73 61 76 95 67 100 93 54↓ 65 85 82 72 66 94 83 90

V. Crujeiras et al. / Data in Brief 4 (2015) 614–621

P

127 128 129

M M M

130 131 132 133 134 135 136 137 138 139

F F M M F F M F F F

140 141 142 143 144 145 146 147 148

F M F M F M F F F

149 150

M M

151

F

3y 6m 11y 8y 1m 2y 10m 5y 7m 2y 11m 3y 4m 1y 6m 23y 9m 28y 1m 22y 7m 10y 4m 17y 2m 19y 19m 8y 2m 13y 4m 19y 11m 27y 9m 12y 7m 10y 4m 18y 5m 42y 1m 11y 5m 23y 5m 25y 4m 21y11m 17y 10m 14y 13y 10y 7m 10y 2m 3y 5m 2y 8m 1y 5m 5y 7m 18y 10m 7y 11m 11y 11m 8y 6m

N N ↑ N N N N N N ↑↑ N N N N

HPA HPA HPA HPA HPA HPA HPA HPA CPKU CPKU CPKU MPKU CPKU MPKU

– – – – – – – – D D D D/T D D

E E E E E E E E E E E E E E

2400 2299 1800 1900 1415 1450 2100 950 235 437 443 922 245 312

198 300 266 97 139 283 180 305 1545 624 516 220 990 882

7.1 7.4 7 7 7 6.3 7.6 6.6 7.6 7.6 6.1↓ 7.7 7.3 7.5

23 20↓ 20↓ 20↓ 17↓ 21 34 18↓ 24 24 21 26 30 35

10.1 9.6 10.4 10.4 10.2 9.3 10.9↑ 10.1 9.5 9.5 8.9 11↑ 9.8 10.2

4.6 4.4 4.1 5.3 5.1 3.9 5.5 5.9 2.5↓ 3.3 3.8 4 6.5↑ 2.2↓

10↓ 20 44 27 17 94 47 10↓ 32 80 212 42 28 41

669 650 495 483 741 435 606 190 421 1289 982 435 363 387

16.2 17.5 13.8 17.5 17 17.5 17.5 15.1 14 17 17 12 5.8 17.5

12↓ 14↓ 29 30 30.5 30 20 29 24 41↑ 28 27 24 33

98 79 74 65 72 62↓ 73 61↓ 109 65 76 71 112 95

81 83 95 106 72 61 70 53↓ 85 69 87 86 75 73

↑↑ N ↑↑

HPA CPKU CPKU

– D D

E E E

2100 182 492

290 282 396

7.5 6.7 7.1

21 32 29

10.1 10.1 9.8

4.6 4.5 4.7

23 44 29

790 394 445

10.4 17.2 11.8

42 ↑ 30 29

70 91 76

93 85 69

N ↑↑ N ↓ ↑ ↑↑ ↑ N N N

CPKU MPKU MPKU CPKU MPKU MPKU CPKU CPKU MPKU MPKU

D D/T D/T D D/T D/T D D/T D/T D/T

E E E E E E L E E E

365 823 922 256 855 2052 452 730 3000 802

516 279 222 195 756 180 735 803 232 436

6.6 6.5 7.5 6.9 7 7.3 7.8 7.4 7.5 7.5

24 21 26 34 29 21 29 31 26 29

8.7 9.4 9.8 9.8 9.6 10.3 9.7 9.9 9.9 9.5

3.4 4.1 3.8 4.3 4.3 3.8 4.2 2.8 3.7 3.4

40 32 55 17 – 72 18 42 30 22

1069 389 437 269 366 420 209 229 394 571

17.5 14 12 10.2 14.7 10.3 17 4.7 11.1 17.5

40 32 27 28 35 38 20 27 28 22

85 74 70 76 116 98 81 69 70 98

85 65 86 74 68 64 88 89 82 72

N N ↑↑ N ↑↑ N N N ↑

CPKU CPKU CPKU MPKU CPKU CPKU CPKU HPA HPA

D D D D/T D D D – –

E E E E E E E E E

413 260 148 1600 264 165 174 1900 2800

204 449 476 245 169 76 248 245 260

7.6 8 7.5 6.8 6.6 6.6 7.2 7.5 7.4

32 37 24 25 22 24 24 24 30

10 10 9.9 10.2 10.4 10.5 10.4 10.1 9.7

4.1 4 4.1 4.3 5.2 4.2 4.9 4.8 4

43 50 29 39 27 25 20 18 17

727 905 605 699 1247 1057 877 382 520

11.1 17.5 13.7 16.1 – – – 10.1 3.9

31 33 27 27 26 24 23 20 31

86 101 113 68 89 82 95 97 86

68 85 67 87 80 80 65 72 70

N N

HPA HPA

– –

E E

1450 2300

235 280

7.5 7.4

21 24

10.8 9.6

4.5 4.9

21 14

730 509

17 8.5

20 20

77 132

75 85

N

HPA



E

1169

270

6.8

21

9.5

4.5

48

356

41.5

20

90

69

619

F M M F F F F M F F M M F F

V. Crujeiras et al. / Data in Brief 4 (2015) 614–621

113 114 115 116 117 118 119 120 121 122 123 124 125 126

620

Table 1 (continued ) P

Sex

Age

BMI

Diag

152 153 154 155 156

M M F F M

1y 6m 3y 3m 23y 2m 37y 3m 16y 5m

N N N ↑ N

HPA HPA CPKU CPKU HPA

Tre. D/ TD Phe tol mg/ T d – – D D –

E E L L E

950 1642 451 299 330

Phe median mM

Prot g/ dL

Prealb mg/ dL

Ca mg/ dL

P mg/ dL

Fer ng/ mL

B12 pg/ mL

Folic ng/ mL

25-OHD ng/ mL

Zn mg/ dL

Se mg/ dL

139 192 1272 576 2000

6.8 6.6 7.4 7.2 7.1

22 22 35 25 22

9.7 9.4 10.8 10.3 10.1

4 5.2 3.6 3.3 3.4

30 19 25 23 22

342 537 362 228 640

9.6 26 4.2 – 15

29 24 23 20 33

78 74 81 85 74

70 64 91 67 87

V. Crujeiras et al. / Data in Brief 4 (2015) 614–621

Characteristics of Hyperphenylalaninaemia patients with altered values of the biochemical parameters studied. P: patient; M: male; F: female; BMI: body mass index; N: normal; ↑overweight, ↑↑ obesity, CPKU: classic phenylketonuria; MPKU: mild-moderate PKU; MHPA: moderate hyperphenylalaninaemia; Tre:Treatment; D: Dietetic; T: BH4 therapy; TD: time of diagnosis; E: Early; L:late; Phe tol: Phenylalanine tolerance; Prot: Total protein total; Prealb: Prealbumin; Fer: Ferritine.

V. Crujeiras et al. / Data in Brief 4 (2015) 614–621

621

Total protein, Ca, P, B12, ferritin, and Zn levels were in the normal range in almost all patients. Nevertheless, Palb was decreased in 34.6% of patients (74% from PKU) being the 94.4% of them below 18 years old and having the 96.3% an adequate adherence to treatment. Se was found to be diminished in 25% of patients, 76.9% of them with classic PKU but only in 2.56% of patients under BH4 treatment. An adequate intake of Ca, P and 25-OHD is necessary for optimal bone accretion but it is not enough for bone normal development because several studies have showed that protein intake deficiency can influence the bone mass [6,7]. Our patients generally had adequate levels of Ca and P, but there were found low levels of 25-OHD in 14% of them. The 25-OHD was found altered in 14% of total patients. No deficiency of folic acid was found in our cohort, but was found to be above the upper limit in 39% of patients, 65.6% with classic PKU and 82% of them with adequate adherence to diet. Patients treated with BH4 presented lower levels of B12 with respect to non-treated (536 vs 694 pg/ mL, p: 0.0094). No other anthropometric parameters were found to be correlated with any variable in our study. We noted in our study a high percentage of PKU patients with good adherence to diet with Prealbumin deficiency and total protein normal. Also a high percentage of PKU patients with Se deficiency had folic acid increase. On the contrary, only el 2.56% de patients under BH4 treatment showed Se deficiency. Appendix A. Supporting information Supplementary data associated with this article can be found in the online version at http://dx.doi. org/10.1016/j.dib.2015.07.026.

References [1] V. Crujeiras, L. Aldámiz-Echevarría, J. Dalmau, I. Vitoria, F. Andrade, I. Roca, R. Leis, A. Fernández-Marmiesse, M.L. Couce, Vitamin and mineral status in patients with hyperphenylalaninemia pii: S1096-7192(15)30029-9, Mol. Genet. Metab. 115 (2015) 145–150, http://dx.doi.org/10.1016/j.ymgme.2015.06.010. [2] J. Vockley, H.C. Andersson, K.M. Antshei, N.E. Braveman, B.K. Burton, J. Mitchell, W.E. Smith, B.H. Thompson, S.A. Berry, Phenylalanine hydroxylase deficiency: diagnosis and management guideline, Genet. Med. 16 (2014) 188–200. [3] W.B. Hanley, A. Feigenbaum, J.T. Clarke, W. Schoonheyt, V. Austin, Vitamin B12 deficiency in adolescents and young adults with phenylketonuria, Lancet 342 (1993) 997. [4] M. Robert, J.C. Rocha, M. van Rijn, K. Ahring, A. Bélanger-Quintana, A. MacDonald, K. Dokoupil, H. GokmenOzel, A.M. Lammardo, P. Goyens, F. Feillet, Micronutrient status in phenylketonuria, Mol. Genet. Metab. 110 (2013) S6–17. [5] J.R. Barretto, L.R. Silva, M.E. Leite, N. Boa-Sorte, H. Pimentel, A.C. Purificação, G. Carvalho, M.I. Fontes, T. Amorim, Poor zinc and selenium status in phenylketonuric children and adolescents in Brazil, Nutr. Res. 28 (2008) 208–211. [6] A. Mirás, M.D. Bóveda, M.R. Leis, A. Mera, L. Aldámiz-Echevarría, J.R. Fernández-Lorenzo, J.M. Fraga, M.L. Couce, Risk factors for developing mineral bone disease in phenylketonuric patients, Mol. Genet. Metab. 108 (2013) 149–154. [7] T.R. Fenton, S.C. Tough, A.W. Lyon, M. Eliasziw, D.A. Hanley, Causal assessment of dietary acid load and bone disease: a systematic review & meta-analysis applying Hill's epidemiologic criteria for causality, Nutr. J. 30 (2011) 10–41.

Micronutrient in hyperphenylalaninemia.

The data presented here are the biochemical parameters of 156 patients with hyperphenylalaninemia. PKU patients, who, in order to maintain optimal ser...
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